Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults.
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Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults. / Dantonello, Tobias M; Int-Veen, Christoph; Harms, Dieter; Leuschner, Ivo; Schmidt, Bernhard F; Herbst, Manfred; Juergens, Heribert; Scheel-Walter, Hans-Gerhard; Bielack, Stefan S; Klingebiel, Thomas; Dickerhoff, Roswitha; Kirsch, Sylvia; Brecht, Ines; Schmelzle, Rainer; Greulich, Michael; Gadner, Helmut; Greiner, Jeanette; Marky, Ildiko; Treuner, Joern; Koscielniak, Ewa.
in: J CLIN ONCOL, Jahrgang 27, Nr. 9, 9, 2009, S. 1446-1455.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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T1 - Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults.
AU - Dantonello, Tobias M
AU - Int-Veen, Christoph
AU - Harms, Dieter
AU - Leuschner, Ivo
AU - Schmidt, Bernhard F
AU - Herbst, Manfred
AU - Juergens, Heribert
AU - Scheel-Walter, Hans-Gerhard
AU - Bielack, Stefan S
AU - Klingebiel, Thomas
AU - Dickerhoff, Roswitha
AU - Kirsch, Sylvia
AU - Brecht, Ines
AU - Schmelzle, Rainer
AU - Greulich, Michael
AU - Gadner, Helmut
AU - Greiner, Jeanette
AU - Marky, Ildiko
AU - Treuner, Joern
AU - Koscielniak, Ewa
PY - 2009
Y1 - 2009
N2 - PURPOSE: To improve risk-adapted therapy for localized childhood soft tissue sarcoma within an international multicenter setting. PATIENTS AND METHODS: Four hundred forty-one patients younger than 21 years with localized rhabdomyosarcoma and rhabdomyosarcoma-like tumors (ie, extraosseous tumors of the Ewing family, synovial sarcoma, and undifferentiated sarcoma) were eligible. Therapy was stratified according to postsurgical stage, histology, and tumor site. In unresectable tumors, treatment was further adapted depending on response to induction chemotherapy, TN classification, tumor size and second-look surgery. A novel five-drug combination of etoposide, vincristine, dactinomycin, ifosfamide, and doxorubicin (EVAIA) was evaluated for high-risk patients, but cumulative chemotherapy dosage and treatment duration were reduced for the remaining individuals as compared with that of the previous trial CWS-86. Hyperfractionated accelerated radiotherapy (HART) was recommended at doses of either 32 or 48 Gy. RESULTS: At a median follow-up of 8 years, 5-year event-free survival (EFS) and overall (OS) survival for the entire cohort was 63% +/- 4% and 73% +/- 4%, respectively (all survival rates in this abstract are calculated and displayed with +/-95% CI). EFS/OS rates by histology were 60% +/- 5%/72% +/- 5% in rhabdomyosarcoma, 62% +/- 10%/69% +/- 10% for Ewing tumors of soft tissues, 84% +/- 12%/90% +/- 10% for synovial sarcoma, and 67% +/- 38%/83% +/- 30% for undifferentiated sarcoma, respectively. Response to one cycle of the five-drug combination EVAIA was similar to that of the four-drug combination VAIA used in CWS-86. Two hundred twelve patients with rhabdomyosarcoma underwent radiation (EFS, 66% +/- 6%); 53 of those patients had a favorable risk profile and received 32 Gy of HART (EFS, 73% +/- 12%). TN classification, tumor site, tumor size, histology, and age were prognostic in univariate analysis. CONCLUSION: Improved risk stratification enabled decreased therapy intensity for selected patients without compromising survival. Intensified chemotherapy with EVAIA did not improve outcome of localized high-risk rhabdomyosarcoma.
AB - PURPOSE: To improve risk-adapted therapy for localized childhood soft tissue sarcoma within an international multicenter setting. PATIENTS AND METHODS: Four hundred forty-one patients younger than 21 years with localized rhabdomyosarcoma and rhabdomyosarcoma-like tumors (ie, extraosseous tumors of the Ewing family, synovial sarcoma, and undifferentiated sarcoma) were eligible. Therapy was stratified according to postsurgical stage, histology, and tumor site. In unresectable tumors, treatment was further adapted depending on response to induction chemotherapy, TN classification, tumor size and second-look surgery. A novel five-drug combination of etoposide, vincristine, dactinomycin, ifosfamide, and doxorubicin (EVAIA) was evaluated for high-risk patients, but cumulative chemotherapy dosage and treatment duration were reduced for the remaining individuals as compared with that of the previous trial CWS-86. Hyperfractionated accelerated radiotherapy (HART) was recommended at doses of either 32 or 48 Gy. RESULTS: At a median follow-up of 8 years, 5-year event-free survival (EFS) and overall (OS) survival for the entire cohort was 63% +/- 4% and 73% +/- 4%, respectively (all survival rates in this abstract are calculated and displayed with +/-95% CI). EFS/OS rates by histology were 60% +/- 5%/72% +/- 5% in rhabdomyosarcoma, 62% +/- 10%/69% +/- 10% for Ewing tumors of soft tissues, 84% +/- 12%/90% +/- 10% for synovial sarcoma, and 67% +/- 38%/83% +/- 30% for undifferentiated sarcoma, respectively. Response to one cycle of the five-drug combination EVAIA was similar to that of the four-drug combination VAIA used in CWS-86. Two hundred twelve patients with rhabdomyosarcoma underwent radiation (EFS, 66% +/- 6%); 53 of those patients had a favorable risk profile and received 32 Gy of HART (EFS, 73% +/- 12%). TN classification, tumor site, tumor size, histology, and age were prognostic in univariate analysis. CONCLUSION: Improved risk stratification enabled decreased therapy intensity for selected patients without compromising survival. Intensified chemotherapy with EVAIA did not improve outcome of localized high-risk rhabdomyosarcoma.
KW - Adult
KW - Humans
KW - Male
KW - Female
KW - Adolescent
KW - Young Adult
KW - dosage
KW - Child
KW - Combined Modality Therapy
KW - Child, Preschool
KW - Infant
KW - Antineoplastic Combined Chemotherapy Protocols therapeutic use
KW - Dactinomycin administration
KW - Dose Fractionation
KW - Doxorubicin administration
KW - Etoposide administration
KW - Ifosfamide administration
KW - Infant, Newborn
KW - Rhabdomyosarcoma therapy
KW - Sarcoma therapy
KW - Sarcoma, Ewing's therapy
KW - Sarcoma, Synovial therapy
KW - Soft Tissue Neoplasms therapy
KW - Vincristine administration
KW - Adult
KW - Humans
KW - Male
KW - Female
KW - Adolescent
KW - Young Adult
KW - dosage
KW - Child
KW - Combined Modality Therapy
KW - Child, Preschool
KW - Infant
KW - Antineoplastic Combined Chemotherapy Protocols therapeutic use
KW - Dactinomycin administration
KW - Dose Fractionation
KW - Doxorubicin administration
KW - Etoposide administration
KW - Ifosfamide administration
KW - Infant, Newborn
KW - Rhabdomyosarcoma therapy
KW - Sarcoma therapy
KW - Sarcoma, Ewing's therapy
KW - Sarcoma, Synovial therapy
KW - Soft Tissue Neoplasms therapy
KW - Vincristine administration
M3 - SCORING: Zeitschriftenaufsatz
VL - 27
SP - 1446
EP - 1455
JO - J CLIN ONCOL
JF - J CLIN ONCOL
SN - 0732-183X
IS - 9
M1 - 9
ER -